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  • Image not available. Systemic lupus erythematosus (SLE) is a disease that predominantly occurs in women.
  • Image not available. The hallmark of SLE is the development of autoantibodies to cellular nuclear components, resulting in chronic inflammatory autoimmune disease. Symptoms and organ involvement depend on the nature of the autoantibodies.
  • Image not available. SLE has a wide spectrum of symptoms and organ system involvement, making therapy highly patient specific. In addition, the signs and symptoms will fluctuate over time.
  • Image not available. The wide spectrum of symptoms and organ system involvement makes pharmacotherapy difficult because therapy must be individualized based on each patient's disease activity.
  • Image not available. There is a paucity of quality evidence for the treatment of SLE except for lupus nephritis.
  • Image not available. Almost all classes of medications, including antiinflammatory and immunosuppressive agents, are reported to cause vasculitis in most major organ systems.

Upon completion of the chapter, the reader will be able to:

  • 1. Describe the epidemiology of systemic lupus erythematosus (SLE).
  • 2. Discuss the potential genetic, environmental, and hormonal basis for SLE etiology.
  • 3. Describe the pathophysiology of SLE.
  • 4. List the organ systems that can be involved for patients with SLE.
  • 5. Identify signs and symptoms of SLE.
  • 6. Differentiate the clinical manifestations between idiopathic SLE and drug-induced SLE.
  • 7. Compare and contrast the adverse effects of the drugs commonly used to treat SLE.
  • 8. Describe the monitoring parameters for the drugs commonly used to treat SLE.
  • 9. Discuss the clinical circumstances when nonsteroidal antiinflammatory drugs are used in the treatment of SLE.
  • 10. Recommend therapy for patients with lupus nephritis.
  • 11. List common side effects of high-dose, long-term corticosteroid use.
  • 12. Discuss therapeutic options for treating SLE during pregnancy.
  • 13. Describe the drug treatment for systemic sclerosis.
  • 14. Describe the drug treatment for polymyalgia rheumatica and giant-cell arteritis.
  • 15. Identify organ systems that can be affected by drug-induced vasculitis.

The collagen–vascular diseases are a heterogeneous group of diseases that can involve the musculoskeletal system, integument, and blood vessels. Each collagen–vascular disease has its own set of diagnostic criteria, although diagnosis can be difficult because of overlapping and nonspecific clinical presentations. The etiology of the various collagen–vascular diseases is often unknown, but the immune system usually is involved in the pathogenesis and manifestations of the disease. Therefore, pharmacotherapy usually includes antiinflammatory agents with or without immunosuppressive drugs.

Although the prevalence of other collagen–vascular diseases may be greater than that of systemic lupus erythematosus (SLE) (e.g., polymyalgia rheumatica), SLE is discussed most extensively in this chapter because it is a major collagen–vascular disease with numerous clinical manifestations, its pharmacotherapy can be complex, and a plethora of data is available on the therapy of SLE. As all the diseases discussed in this chapter have an immune-mediated pathogenesis, the therapeutic principles of SLE can be applied to other autoimmune collagen–vascular diseases. The collagen–vascular diseases discussed include systemic sclerosis, polymyositis/dermatomyositis, polymyalgia rheumatica, and drug-induced vasculitis; these were chosen because they are seen in general practice.

SLE is a fluctuating ...

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